Department of Endocrinology, Skåne University Hospital, SE-221 85 Lund, Sweden.
J Clin Endocrinol Metab. 2013 Nov;98(11):4219-26. doi: 10.1210/jc.2013-2415. Epub 2013 Sep 12.
During GH therapy for 2.3-9.6 years, male adult-onset GH-deficient patients with a diagnosis of a nonfunctioning adenoma have no increased all-cause mortality. However, women with adult-onset GH deficiency (GHD) are still at slightly higher risk. This general improvement in mortality is due to a more contemporary regimen of cardiovascular drugs, a refinement of surgical procedures, besides the introduction of GH therapy improved hormone replacement regimens with lowered glucocorticoid replacement, updated approaches of sex steroids for women, and less use of cranial radiotherapy. The underlying disease is the most important predictor for mortality: eg, a craniopharyngioma, malignant causes of hypopituitarism, previous Cushing's disease, and the presence of diabetes insipidus/aggressive tumors. The main cause of increased mortality was cerebrovascular diseases and infectious/respiratory diseases in ACTH-deficient patients. Furthermore, there was a significant impact of young age at disease onset and of death from secondary brain tumors, with a higher risk after cranial radiotherapy. Reports on four cohorts of GH-treated childhood-onset GHD patients have been published. Two of them included only patients with idiopathic isolated GHD, neurosecretory dysfunction, idiopathic short stature, or being born short for gestational age. Increased mortality in circulatory disorders, ill-defined diseases, and bone cancer were recorded in one study, but not in the other smaller study, where suicide and accidents caused the majority of deaths. A third childhood-onset GHD cohort included patients with a background of malignant tumors, craniopharyngioma, pituitary adenomas, pituitary aplasia/hypoplasia, and trauma. An increase of all-cause mortality was recorded in both males and females. The fourth cohort included isolated GHD and idiopathic short stature (60%), but also diagnosis of chronic renal failure and Turner's syndrome. In these latter studies, an underlying serious condition was the most important factor for death, with central nervous system tumors (recurrent or new tumor) being the leading cause of mortality.
在接受 GH 治疗 2.3-9.6 年后，被诊断为无功能腺瘤的男性成年期 GH 缺乏症患者的全因死亡率没有增加。然而，女性成年期 GH 缺乏症（GHD）患者的风险仍然略高。这种死亡率的普遍改善归因于心血管药物的现代治疗方案、手术方法的改进，以及 GH 治疗的引入，改善了激素替代方案，降低了糖皮质激素替代，更新了女性性激素的治疗方法，以及减少了颅放疗的应用。基础疾病是死亡率的最重要预测因素：例如颅咽管瘤、垂体功能减退的恶性原因、既往库欣病和尿崩症/侵袭性肿瘤的存在。ACTH 缺乏症患者死亡率增加的主要原因是脑血管疾病和感染/呼吸道疾病。此外，发病年龄较轻和继发脑肿瘤死亡是导致死亡率增加的重要原因，颅放疗后风险更高。已经发表了四份 GH 治疗儿童期 GHD 患者的队列研究报告。其中两份仅包括特发性孤立性 GHD、神经分泌功能障碍、特发性身材矮小或出生时胎龄较小的患者。一项研究记录了循环系统疾病、定义不明确的疾病和骨癌的死亡率增加，但另一项较小的研究中没有记录到这一点，其中自杀和意外事故是导致死亡的主要原因。第三个儿童期 GHD 队列包括患有恶性肿瘤、颅咽管瘤、垂体腺瘤、垂体发育不全/发育不良和创伤背景的患者。男性和女性的全因死亡率均有所增加。第四个队列包括孤立性 GHD 和特发性身材矮小（60%），但也包括慢性肾功能衰竭和 Turner 综合征的诊断。在这些研究中，基础严重疾病是死亡的最重要因素，中枢神经系统肿瘤（复发或新肿瘤）是导致死亡的主要原因。
